Atresia of the bilious courses – the anomaly of development of biliary tract which is expressed in violation of passability of intra hepatic or/and extra hepatic bilious channels. The atresia of the bilious courses is shown by persistent jaundice, decolouration of excrements, intensive dark coloring of urine, increase in a liver and spleen, development of portal hypertensia, ascites. The atresia of the bilious courses is distinguished by means of ultrasonography, a holangiografiya, a stsintigrafiya, a laparoscopy, biochemical tests, a punktsionny biopsy of a liver. At an atresia of the bilious courses expeditious treatment is shown (gepatiko-, a holedokhoyeyunostomiya, a portoenterostomiya).
Atresia of the bilious courses
Atresia of the bilious courses (biliarny atresia) – the heavy congenital malformation which is characterized by a partial or full obliteration of a gleam of the bilious courses. The atresia of the bilious courses meets in pediatrics and children's surgery approximately in 1 case on 20000-30000 childbirth, making about 8% of all defects of internals at children. The atresia of the bilious courses represents extremely dangerous state demanding early identification and urgent expeditious treatment. Without rendering the timely surgical help children with an atresia of the bilious courses perish within the first months of life from esophageal bleeding, a liver failure or infectious complications.
Reasons of an atresia of the bilious courses
The reasons and pathogenesis an atresia of the bilious courses are up to the end not clear. In most cases at an atresia bilious channels are created, however their passability is broken owing to an obliteration or the progressing destruction. Most often pre-natal infections (herpes, a rubella, a cytomegalic inclusion disease, etc.) or neonatal hepatitis become the reason of an obstuktivny holangiopatiya. Inflammatory process is caused by injury of hepatocytes, an endoteliya of the bilious courses with the subsequent intracellular holestaz and fibrosis of the bilious courses. Less often the atresia of the bilious courses is connected with ischemia of channels. In these cases an atresia of the bilious courses it will not be obligatory to have congenital character, and can develop in the perinatal period owing to the progressing inflammatory process.
The true atresia of the bilious courses comes to light less often and is connected with violation of primary laying of bilious ways in the embryonic period. At the same time in case of violation of laying of a hepatic diverticulum or the sewerage of disteel departments of bile-excreting system the atresia of the intra hepatic bilious courses develops, and at violation of formation of proximal departments of bile-excreting system - an atresia of extra hepatic bilious channels. The gall bladder can be the only link of external biliary tract or be absent completely.
Approximately at 20% of children the atresia of the bilious courses is combined with other anomalies of development: congenital heart diseases, incomplete turn of intestines, aspleniya or polispleniya.
Classification of an atresia of the bilious courses
The following kliniko-morphological forms belong to anomalies of development of biliary tract: hypoplasia, atresia, violation of branching and merge, cystous changes of the bilious courses, anomalies of a holedokh (cyst, stenosis, perforation).
On localization distinguish the extra hepatic, intra hepatic and total (mixed) atresia of the bilious courses. Taking into account the place of obstruction of bilious channels allocate a korrigiruyemy atresia of the bilious courses (a neprokhodiyomost of the general bilious or general pecheyonochny channel) and a nekorrigiruyemy atresia of the bilious courses (full replacement of channels in liver gate with fibrous fabric). Localization and level of obstruction of bilious channels are the defining factors influencing the choice of the technology of surgical intervention.
Symptoms of an atresia of the bilious courses
Children with an atresia of the bilious courses usually are born full-term with normal anthropometrical indicators. For the 3-4th days of life they develop jaundice, however unlike a tranzitorny giperbilirubinemiya of newborns, jaundice at an atresia of the bilious courses remains is long and gradually accrues, giving to integuments of the child a saffron-yellow or greenish shade. Usually the atresia of the bilious courses is not distinguished in the first days of life of the child, and the newborn is discharged from maternity hospital with the diagnosis "prolonged physiological jaundice".
As the characteristic signs of an atresia of the bilious courses which are present from the first days of life of the child serve the akholichny (decoloured) chair and urine of rich dark ("beer") color. Preservation decoloured a calla within 10 days indicates an atresia of the bilious courses.
By the end of the first month of life the loss of appetite, deficiency of weight, an adinamiya, a skin itch, a delay of physical development accrues. In the same time increase in a liver is noted, and then – spleens. Gepatomegaliya is a consequence of holestatichesky hepatitis against the background of which biliarny cirrhosis, ascites, and by 4-5 months - a liver failure quickly develops. Violation of blood supply of a liver promotes formation of portal hypertensia and, as a result, - to a varicosity of a gullet, a splenomegaliya, emergence of hypodermic venous network on a forward belly wall. At children with an atresia of the bilious courses the hemorrhagic syndrome is noted: melkotochechny or extensive hemorrhages on skin, bleedings from the funic rest, esophageal or gastrointestinal bleedings.
In the absence of timely surgical treatment children with an atresia of the bilious courses usually do not live till 1 year, perishing from the liver failure, bleedings which joined pneumonia, cardiovascular insufficiency, interkurentny infections. At an incomplete atresia of the bilious courses some children can live up to 10 years.
Diagnostics of an atresia of the bilious courses
It is extremely important that the atresia of the bilious courses was revealed neonatology or the pediatrician as soon as possible. Persistiruyushchy jaundice and an akholichny chair have to suggest children's experts an idea of presence at the child of an atresia of the bilious courses and force to take necessary measures to specification of the diagnosis.
From the first days of life in blood of the newborn levels of biochemical markers of a liver, first of all, of bilirubin (in the beginning due to increase in direct fraction, later – due to prevalence of indirect bilirubin), alkaline phosphatase, gamma - transferases increase. Gradually anemia and thrombocytopenia develops, PTI decreases. At an atresia of the bilious courses in Calais there is no child .
Ultrasonography of a liver, spleen and bilious ways allows to estimate the sizes of bodies and structure of a parenchyma, a condition of a gall bladder, out of - and intra hepatic bilious channels, a vorotny vein. Usually at an atresia of the bilious courses the gall bladder is not defined or visualized in the form of a tyazh, the general bilious channel also does not come to light. As informative methods for specification of the diagnosis serve a chreskozhny chrespechenochny holangiografiya, MR-holangiografiya, the static stsintigrafiya of a liver, a dynamic stsintigrafiya of gepatobiliarny system.
Final confirmation of an atresia of the bilious courses can be reached when performing a diagnostic laparoscopy, intraoperative holangiografiya, liver biopsy. The morphological research of a bioptat promotes specification of nature of damage of hepatocytes and conditions of intra hepatic bilious channels.
The atresia of the bilious courses at children should be differentiated with jaundice of newborns, congenital gigantokletochny hepatitis, mechanical obstruction of bilious ways mucous or bilious traffic jams.
Treatment of an atresia of the bilious courses
Important condition of success of treatment of an atresia of the bilious courses is carrying out surgical intervention aged up to 2-3 months as at more advanced age operation is, as a rule, unsuccessful because of irreversible changes.
In case of impassability of the general bilious or hepatic channels and existence of suprastenichesky expansion imposing of a holedokhoenteroanastomoz (holedokhoyeyunostomiya) or a gepatikoyeyunoanastomoza (gepatikoyeyunostomiya) with a loop of a lean gut is made. The full atresia of the extra hepatic bilious courses dictates need of performance of a portoenterostomiya across Kasai – imposings of an anastomoz between gate of a liver and a small intestine. Serve as the most frequent postoperative complications postoperative holangit, liver abscesses.
At the expressed portal hypertensia and recurrent gastrointestinalny bleedings portokavalny shunting can be carried out. In case of the progressing liver failure and a gipersplenizm the issue of transplantation of a liver is resolved.
Forecast of an atresia of the bilious courses
In general the forecast of an atresia of the bilious courses very serious as tsirrotichesky changes of a liver have the progressing character. Without the surgical help children are doomed to death within 12-18 months.
It is very important to reveal clinical manifestations of an atresia of the bilious courses (persistent jaundice, akholichny kcal) in the neonatal period and to carry out early surgical treatment. In most cases imposing of holedokhodigestivny anastomoz or hepatic anastomoz is capable to prolong the child's life for several years and to delay need of transplantation of a liver.